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Monday, January 30, 2012

This bumpy, dusty, desolate road of Shwachman Diamond Syndrome has been no picnic the last little while. I need to a runaway truck ramp or better yet a rest area of some sort.

The past several months have been harder than usual. I have been struggling with depression, something I have been able to avoid for many years but now, I find myself trying to hide that and keep a happy front. (I guess the cat's out of the bag now!) So many times in the last week, I have been reminded that we are too big, too overwhelming and unbelievable for some people to handle, even some medical providers. In a moment of weakness, I let my guard down and let out a little more emotion than usual. They didn't like it. They let me know in no uncertain terms that I was annoying them. "Gosh, I'm sorry, please accept my apology for sharing with you some of my worries and expecting to get a little emotional support." Silly me. Silly genetic problems.

My kids need to see their happy mom, in the kitchen making a lovely pancake breakfast to send them off for the day. They need warm cookies, straight from the oven and me standing there with a beaming smile from ear to ear as I cheerfully welcome them home after school. They need me to have the answers, know the drill and set things straight. They are getting tired of the tears for no apparent reason, the 'maybe tomorrow, I'm tired' answers and the time to time struggle with patience.

Well, I am human. I know, surprise! Not superhuman, not mother of the year, just me. Just me who sometimes gets caught off guard with the fact that I am a mother. Not just a mother but a mother to three sick kids! A few weeks ago, I stood at the kitchen sink with the mindless task of peeling potatoes and Sam came in and said more than once, "Mom" I heard him but it didn't register that he was talking to me until he poked me and said again, "MOM!" For a split second I looked around wondering who he was talking to! Oh ya, that's me, I'm the mom...duh...how did that happen? Who died and left me in charge? Some days, I don't want to be the mom. It's hard. It's hard to be the barometer of every one's happiness.

Sunday, January 29, 2012

Well, now I know where I stand...my kids don't trust me! After all I've done...
Spencer spent Saturday snowboarding. It has become his usual weekend activity and my day to stress and worry and therein, lies the problem. I try not to and I have gotten better in the recent years to not worry so much about my kids but it's a learning curve that is pretty steep.

Spencer came home Saturday night to get some things. When I heard him open the garage, I was excited to see him. I greeted him at the door with a hug and kiss. I asked him how his day was and this was his response...
"Hey, what's left on our insurance deductible?" This sounded fishy...He made up some story about Shelbie asking him.

I didn't believe him so I texted Shelbie, she had no idea what I was talking about so I called Spencer on it. He said he was just kidding around and wanted to tease me. That was not a funny joke and I knew he was up to something so I kept pressing for information. Then he told me that he had a health report due Monday and needed a brace as a visual aid. That sounded good until he told me the report was for History class.

It took forever before Spencer would spill the beans. Finally, he told me he took a 20' jump but landed on his back and when he landed, the edge of his snowboard caught and flipped him head over heels and he landed on his face. He was afraid if he told me how the injury happened, I would freak out and not let him go snowboarding again.

I felt bad that he didn't trust me enough to tell me so I could get him some help. Spencer is always trying to protect me. He downplays everything that happens to him. It would be hard to be a kid in this family. No matter how much I try to keep the stress to a minimum around here, it's not easy. I feel bad that he feels like he can't tell me when bad things happen to him. So, I will have to work on supporting him more emotionally and he will have to work on his lying and trusting me!

We will have to see how the next few days turn out for him. He says it hurts worse than when he broke it last year. For now, we are tackling it with Advil and ice, along with the back brace he wore last Spring when he broke his back.

It's really hard for me to curtail his activities when he is feeling like his time is running out. At least time when he can do the things he loves. Not a day goes by anymore that he doesn't feel sick and extremely tired but he refuses to slow down. Even though I have dealt with Shwachman Diamond for 19 years now, I still can't imagine how it feels to be them.

It's been 6 months since their last bone marrow biopsy and our doc asked me about getting the next one scheduled for this month but I just can't bring myself to making the call. They are suppose to be getting them every 6 months now because of all the abnormalities and decreasing cellularity...ahhh, tired. Who knew life could get so complicated.

Wednesday, January 25, 2012

Shelbie's transfusion last week ended up being pretty rough. I am starting to develop a theory on the side effects. It seems when her counts her low, the side effects are bad. When her counts are higher, the side effects are manageable.

We spent the weekend just trying to manage pain, extreme pain that even affected her eyes. I was constantly rotating ice packs to wrap her head in. She couldn't sleep so that meant I was pretty much missing out on some zzz's as well. This week, it has caught up to me and I am wiped out. It seemed like every hour I would say to her, "Let's just get through this hour. If things aren't better in one hour, we will go to the ER." Surprisingly, things would ease up and she could muscle through. A few hours later, I would play the 'hour' card again. We were able to avoid the ER which was great.

Last night, she finally turned a corner and today has been a good day for her. Sam on the other hand...

Sam was awakened in the early morning hours with his entire body trembling. It was pretty scary. I heard someone running water and decided to check it out. Sam was standing by the sink throwing cold water on his face. He was white as a ghost and shaking uncontrollably and super weak. He had no other symptoms. I kept him home today to keep an eye on him.

I think he had a mito crash. He's been really pushing himself the last few weeks physically and Tuesday night, he spent a couple of hours practicing his acrobats at Open Gym. I think his body was completely spent. He slept most of the day but seems to be doing better.

That's where we are at this week. I was surprised that Sam is starting to show symptoms of mito. He's been my stable kid for the past year. I hope this isn't the beginning of a new trend.

Sunday, January 22, 2012

While I was laying on the Hidascan table getting the scans of my gallbladder, the technician asked, "Do you have any stress in your life?"

"hmmmm...let me think...No, nothing I can put my finger on. I don't have much to be stressed about." was my response, completely tongue in cheek.

He took me seriously though and said, "Well, that's good. You don't need stress compounding the problem."

"Ya, for sure, that would stink."
Inside, I wasn't sure if I should cry because not a second goes by that I don't feel stressed and worried or if I should laugh because the question itself seemed just so trivial and trite compared to what I am dealing with.

Oh well, for you fellow people stressed, stop that and check out what stress can do to you! Yes, I'm pretty much doomed.

Friday, January 20, 2012

What a day! I was told it could be two weeks before Shelbie would be able to get her IVIG because of communication problems between the hospital, nurses at the oncology clinic and our insurance company. Apparently, our doctor was unaware of the situation because he kept getting misinformed so when I called the office in a bit of a mood on Thursday, he finally realized there was a problem.

He spent the early morning hours Friday, arguing with our insurance company and by 10:00am, he had convinced them to authorize one IVIG treatment and we will need to continue the fight for more. Our doc wanted to see Shelbie before going to the hospital which was good because she has not been feeling well. After a thorough exam it became clear that she has other problems brewing.

Her counts were really low and she is pretty neutropenic meaning her white count and immature white cells called Neutrophils are low as well. She is still fighting the same Adenovirus that she got last JULY!! Yes, the same one. IVIG calms it all down for a couple of weeks but once that is out of her system, the virus raises its ugly head again. On top of all that, her pancreas is acting up and she is suffering with malabsorption so we are starting Pancreatic enzymes back up. That adds up to about 12 more pills a day. Hopefully it will help, she has lost some weight this week so hopefully these new meds will help.

The Oncologist isn't very hopeful that her immune system will ever come back. Today he said she is responding just like his Common Variable Immune Deficiency patients. They get a virus and never recover and only IVIG keeps it at bay. That wasn't very fun to hear. At any rate, we need to get things under control now.

Thursday, January 19, 2012

Today has been a very hard day as I come to terms with how hard it has been raising my kids with Shwachman Diamond and all the other problems they face. The realization of this hit me in the strangest of ways.

I am a baby. When I feel out of sorts or sick with anything other than the usual cold or flu, I get really anxious. I'm not sure why but I think it may have something to do with the fact that I am the mom. I am the caretaker, the caregiver, the housekeeper, the cook, the psychologist, the PA, the go to girl, the resident, kiss is is all better, the one who keeps the train on the tracks, I'm in the bottom of the boat, working the oars and straightening the deck chairs. Not to mention, I am the breadwinner regardless of how small and stale the 'bread' is.

Tuesday, I developed a really bad pain just under my ribs. I tried to ignore it. By Wednesday, it was really bad, a nagging pain I couldn't get away from and then a bad headache started and I am not a headachey kind of girl. Wednesday, late afternoon, I couldn't get comfortable, the pain was filling my insides so I drove straight to my doctor's in a nearby town. It didn't take him long to assess me with what he believes is a gallbladder attack. Today, I will be undergoing some further testing in the city to confirm what he thinks.

I have been stressing about this. I hope it is just a gallbladder problem and not something bigger. I worry about when to have surgery, if I can even afford to have surgery. With an $11,000 deductible, it pretty much sucks. The only income I have these days comes from my physical labor jobs of housecleaning. Surgery means I won't be able to clean for a couple of weeks and that translates into no money but a huge hospital bill. Of course, there is the random thought about what happens if something happens to me? Who takes care of my kids? Who pays the bills?

I hate setbacks like this. It makes me feel like a really bad mom too! Ya know, because I whine and complain when I am sick and go straight to the doctors but when my kids complain, I try every possible thing before taking them in. When Spencer had his gallbladder problem a year ago, that poor kid went nearly a month before getting a diagnosis and surgery. I feel really bad now because the pain is bad, at least the pain I am feeling and he just dealt with it. I don't know that I will ever get a handle on dealing with SDS and Mito. It's so frustrating to me.

Shelbie was suppose to be admitted today for her transfusion but once again, nobody acted on the preauthorization I asked them to start back in November so her treatments wouldn't be interrupted. Now I have a girl with an eye infection, sore throat, cough and congestion and if it turns into something worse than that, I will be so angry! Sometimes, I think they look at us like we are making this crap up. I never feel like we are respected very much and that is starting to wear on me.

Oh well, it's off to be poked and prodded for me. I seriously don't know how my kids do it! I have just added a whole other dimension of respect for them.

Sunday, January 15, 2012

So, how do you like the new banner? To me, it feels like a breath of fresh air. Last year was a year of chaos and stumbling from one crisis to another. By the time December came, I wasn't sure I was going to make it to this year without a new, white, slim fitting jacket with extra long sleeves and silver buckles! Yes, I felt like I was going crazy! Most days, I am still crazy, some things will never change but I feel like I have a little more breathing room.

Life is hard. Life is harder with Shwachman Diamond Syndrome and Mitochondrial Disease and Secondary Immune Deficiency. Really, when I think about it, nothing has changed physically from December to January. We are still preparing for Shelbie's infusion this week. For the last several days, she has had inflammed eyes off and on, sore throat, cough and some GI issues but it has all somehow melded more seamlessly into the day. Sadly, it has become like a weather report we hardly pay attention to. That sounds rude doesn't it? Sounds like I don't care. I'm sure there are times when my kids feel that way too. I do care, I care too much and that's why I am losing my mind.

Every problem I have faced in life had an exit strategy. With enough planning, working, organizing, finessing, sweat, blood and tears, I was able to find a way out from under the crushing force of life. This whole world of chronic illness is totally out of my control! I am still trying to figure out how to live with it, watch it all go down. It's frustrating but then again, that isn't breaking news.

So, here's to another year...another year of plasma transfusions, daily nausea, fatigue, infections and whatever else decides to sneak up on us from behind.

Friday, January 13, 2012

I have been spending alot of my time lately reflecting on the past, the very distant past. There must be a reason for my pondering though I'm not really sure about all the 'whys' of it yet. It's kind of like those days when you think about an old friend and then the next day, they call! Everyday, a little thought, a phone call, a story, all reminding me of where I've been.

When I was born, my hips were dislocated in a bad way. Congenital Hips, they called it. My little baby legs were turned almost backwards. I was put in some pretty funky braces and then my parents were told the odds of me ever walking again were slim.

Earlier this week, I was helping Shelbie with some things in her studio and found a tote box back in the corner of the basement, sitting all alone. I opened it up and there on the top were the braces I wore as a baby. They look more like instruments of torture. I guess in a lot of ways they were. They had the foreboding task of turning my hips around. For months and months, this was the accessory complimenting my little baby girl wardrobe.

The white contraption strapped to my back and my legs went through the little leg holes. The long red bar screwed in to my orthopedic shoes. I must have had long legs cause that's quite the spread.

My stylish orthopedic shoes

Notice the little screw holes in the bottom of the shoes.

Life was crazy for my family back then. While I was caught up in this mess, my mom was fighting cancer. Sometimes, life's like that. This was my introduction to the medical world. Sometimes, I think about these early days and the miracle it is that I am walking, even that I could have babies. It has been a good reminder that a doctor doesn't get the last word, God does.

Now, when I look at my kids, all the odds they've beaten, I am reminded again that they will live out their days, every one of them and not one day less no matter what the doctors think or how grim it may get.

Thursday, January 5, 2012

You know when I don't post for a week or so, things are okay or at least status quo. I hate to say how much I love the break from health problems because whenever I mention how quiet things are, you know what hits the fan and I end up eating my words. I would rather not nosh on that!

All in all, I can't complain. We have had the usual issues like daily nausea, extreme fatigue and Shelbie is fighting an eye infection and cold. Not fun but the infusion is coming soon so that will help. Spencer's petechiae has cleared up and were able to avoid a big infection with the help of fast acting antibiotics. He is still trying to think of a Wish and the deadline is ticking for him to decide. I think the wish granters will be coming by in a couple of weeks. We decided to wait until after the holidays and one of our sweet wish granters had surgery yesterday so I thought we should let her recuperate before getting to work on behalf of Spencer.

We started this year with a positive event that I'm not sure I mentioned. Shelbie was awarded Social Security Disability. We got the call the day before Christmas Eve and it flooded me with mixed emotions. I felt like a huge burden had been lifted. I was anticipating having to pay for this upcoming infusion with pennies from Heaven...(I guess we are actually because it is nothing short of a miracle that she was approved so fast.0 I have to pay private insurance because I don't have a job and so I can only afford a policy with a huge deductible...to the tune of $11,000. That would take me years to pay off but I would have done anything to make sure she got her treatment.

It's almost unheard of for someone to get approved in just two months from the time I applied. After the relief washed over me, I felt sad that there was no question that she is disabled enough that she can't support herself. I never wanted this for my kids. I asked the case worker how this happened so fast. He said, "Well, your paperwork was thorough and all of the her doctors confirmed the information you gave us. There was no question she is a very sick girl." Blech... that's never a fun thing to hear.

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"For a small moment have I forsaken thee, but with great mercies will I gather thee...I hid my face from thee for a moment, but with everlasting kindness will I have mercy on thee, saith the Lord thy Redeemer...For the mountains will depart and the hills shall be removed, but my kindness shall not depart from thee, neither shall the covenant of my peace be removed, saith the Lord that hath mercy on thee.

Oh thou afflicted, tossed with tempest, and not comforted! Behold, I will lay thy stones with fair colors, and lay thy foundations with sapphires. And I will make thy windows of agates and thy gates of carbuncles, and all thy borders of pleasant stones...great shall be the peace of thy children. Thou shalt be far from oppression for thou shalt not fear, and from terror for it shall not come near thee." 3 Nephi 22:7-14